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Topic: Alexander disease


In the News (Sat 25 May 13)

  
  Alexander Disease
Alexander disease is a rare, genetic, degenerative disorder of the nervous system.
It is one of a group of genetic disorders called the leukodystrophies that affect growth of the myelin sheath, the fatty covering--which acts as an insulator--on nerve fibers in the brain.
Alexander disease, which affects mostly males, usually begins at about 6 months of age.
healthlink.mcw.edu /article/921383447.html   (217 words)

  
 CHARCOT-MARIE-TOOTH DISEASE   (Site not responding. Last check: 2007-10-25)
Charcot-Marie-Tooth disease is an inherited degenerative disorder of the central and peripheral nervous system that results in muscle atrophy and loss of proprioception in the affected areas (Alexander and Johnson, 1987).
Charcot-Marie-Tooth disease is usually an autosomal dominant trait which initially manifests in the foot and legs, and subsequently may progress to the hand and forearm.
Alexander and Johnson (1989) postulate that the unopposed strength of the Peroneus Longus, responsible for the medial plantarflexion deformity, contributes to a valgus deformity of the forefoot when the rearfoot is unloaded and held in a neutral position.
www.podiatry.curtin.edu.au:16080 /encyclopedia/ciecle   (3901 words)

  
 About Alexander's Disease
Alexanders disease belongs to a group of progressive neurological disorders in which the destruction of white matter in the brain is accompanied by the formation of fibrous, eosinophilic deposits known as Rosenthal fibers.
Alexanders disease begins in infancy, affects mostly males, and results in retarded development and dementia.
Alexanders disease is thought to be an autosomal recessive disorder which can affect both males and females.
www.dylanfreeman.20m.com /about.html   (375 words)

  
 Alexander Disease   (Site not responding. Last check: 2007-10-25)
Alexander disease is a rare, genetically determined degenerative disorder of the central nervous system.
It is one of a group of disorders known as the leukodystrophies, diseases that result from imperfect growth or development of the myelin sheath, the fatty covering that acts as an insulator around nerve fibers in the brain.
In Alexander disease, the destruction of white matter in the brain is accompanied by the formation of fibrous protein deposits called Rosenthal fibers.
www.clevelandclinic.org /health/health-info/docs/1200/1245.asp   (443 words)

  
 Alexander Disease Bibliography by Date
Nielsen,A.L., Jorgensen,P., and Jorgensen,A.L. Mutations associated with a childhood leukodystrophy, Alexander disease, cause deficiency in dimerization of the cytoskeletal protein GFAP.
Rodriguez, D. Infantile Alexander disease: Spectrum of GFAP mutations and genotype-phenotype correlation (vol 69, pg 1134, 2001).
Iwaki, A., Iwaki, T., Goldman, J. E., Ogomori, K., Tateishi, J., and Sakaki, Y. Accumulation of alpha B-crystallin in brains of patients with Alexander's disease is not due to an abnormality of the 5'- flanking and coding sequence of the genomic DNA.
www.waisman.wisc.edu /alexander/bib-date.html   (3293 words)

  
 Alexander Disease - Treatment and Symptoms
Alexander disease is a rare disorder of the nervous system that usually begins during infancy or early childhood.
Alexander disease is considered one of the leukodystrophies, a group of disorders where the primary abnormality is the inability to maintain the fatty covering (myelin) that insulates nerve fibers in the brain.
It is one of a group of disorders known as the leukodystrophies, diseases that result from imperfect growth or development of the myelin sheath, the fatty covering that acts as an insulator...
goldbamboo.com /topic-t3191.html   (466 words)

  
 ALEXANDER DISEASE: Contact a Family - for families with disabled children: information on rare syndromes and disorders   (Site not responding. Last check: 2007-10-25)
Alexander disease is a rare genetic, degenerative disorder of the nervous system with effects on the mid brain and cerebellum; in the most common infantile form of the disease, the frontal white matter is involved.
In Alexander disease, the growth of the white matter of the brain, or myelin sheath, is affected.
In these cases of Alexander disease the inheritance pattern is unclear and has yet to be subject to molecular analysis of the GFAP gene.
www.cafamily.org.uk /Direct/a31.html   (1033 words)

  
 The clinicopathological spectrum of Rosenthal fibre encephalopathy and Alexander's disease: a case report and review of ...   (Site not responding. Last check: 2007-10-25)
Alexander’s disease in an adult: clinicopathologic study of a case and review of the literature.
Hereditary adult-onset Alexander’s disease with palatal myoclonus, spastic paraparesis, and cerebellar ataxia.
Infantile Alexander disease: spectrum of GFAP mutations and genotype-phenotype correlation.
jnnp.bmjjournals.com /cgi/content/full/74/6/807   (2047 words)

  
 Alexander disease definition - Medical Dictionary definitions of popular medical terms
The infantile form of the disease is characterized by megalencephaly (an abnormally large head), seizures, spasticity and developmental retardation.
Mutations in the gene for GFAP (glial fibrillary acidic protein) cause Alexander disease, the first known example of a primary genetic disorder of astrocytes, one of the major cell types in the vertebrate CNS.
Alexander disease is classified as one of the leukodystrophies, the diseases of the white matter of the brain.
www.medterms.com /script/main/art.asp?articlekey=13493   (319 words)

  
 Mutation Key To Alexander Disease
The illness is one of a family of diseases in which the myelin sheath, which protects nerve cells, is damaged.
While the origin of the disease, first described in 1949, was thought to be genetic, that suspicion was not confirmed until now.
The researchers concluded that GFAP abnormality caused by a genetic mutation is the major cause of Alexander disease.
www.applesforhealth.com /keyalex2.html   (651 words)

  
 Genetic basis of Alexander disease discovered
Because of the rarity of the disease, it took nearly two years to assemble enough cases from international sources to complete the study.
Alexander disease is in a family of disorders called leukodystrophies in which abnormalities arise in the myelin sheath, a protective insulation that covers nerves.
The Nature Genetics paper confirmed that mutations in a gene called GFAP - or glial fibrillary acidic protein - are associated with nearly all cases of Alexander disease.
www.eurekalert.org /pub_releases/2001-01/UoW-GboA-0101101.php   (529 words)

  
 Alexander Disease Information
It is one of a group of genetic disorders called the leukodystrophies that affect growth of the myelin sheath - the fatty covering which acts as an insulator - on nerve fibers in the brain.
Alexander disease, which affects mostly males, is usually nonfamilial and begins at about 6 months of age.
The prognosis for individuals with Alexander disease is usually poor.
medicalwisdom.com /health/disorders/alexand_doc.htm   (276 words)

  
 Alexander Disease: Diagnosis with MR Imaging -- van der Knaap et al. 22 (3): 541 -- American Journal of Neuroradiology
Alexander disease, in one patient at an early stage and in the
of Alexander disease was obtained in one of the 14 patients.
Alexander disease: Ventricular garlands and abnormalities of the medulla and spinal cord
www.ajnr.org /cgi/content/full/22/3/541   (5361 words)

  
 Medical Dictionary: Alexander Disease - WrongDiagnosis.com
Alexander Disease: Alexander Disease is listed as a type of (or associated with) the following medical conditions in our database: leukodystrophy, Brain conditions, Genetic Disease
Alexander Disease: Alexander disease is a rare, genetic, degenerative disorder of the nervous system.
It is one of a group of genetic disorders called the leukodystrophies that affect growth of the myelin sheath — the fatty covering which acts as an insulator — on nerve fibers in the brain.
www.wrongdiagnosis.com /medical/alexander_disease.htm   (191 words)

  
 Alexander Disease Bibliography by Topic
Jacob, J., Robertson, N. J., and Hilton, D. The clinicopathological spectrum of Rosenthal fibre encephalopathy and Alexander's disease: a case report and review of the literature.
Li, R., Messing, A., Goldman, J.E. & Brenner, M. GFAP mutations in Alexander disease.
Messing, A., Goldman, J. E., Johnson, A. B., & Brenner, M. Alexander disease: new insights from genetics.
www.waisman.wisc.edu /alexander/bib-topic.html   (414 words)

  
 Alexander's Disease, a Leukodystrophy
We have a 2 yr old son with Alexander's Disease.
We would love to meet others who are dealing with this disease or have dealt with it.
Hi Tasos, my name is Elena, I have a daugther with alexander's too, she was diagnosed at 5 yrs old and she is 19 yrs now and doing well, every day we see improvements and Dimitri will do it too,...
groups.msn.com /AlexandersDiseaseaLeukodystrophy   (198 words)

  
 Alexander Disease / Library / The Family Village   (Site not responding. Last check: 2007-10-25)
Alexander Disease / Library / The Family Village
Leukodystrophies are a group of genetic nervous system disorders affecting the myelin sheath, which insulates the axon through which nerve impulses are conducted.
From the Alexander Disease Project at the Waisman Center, University of Wisconsin
www.familyvillage.wisc.edu /lib_alex.html   (105 words)

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